Type |
Details |
Score |
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
411
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
396
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
437
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Publication |
First Author: |
Alcedo J |
Year: |
1996 |
Journal: |
Cell |
Title: |
The Drosophila smoothened gene encodes a seven-pass membrane protein, a putative receptor for the hedgehog signal. |
Volume: |
86 |
Issue: |
2 |
Pages: |
221-32 |
|
•
•
•
•
•
|
Protein Domain |
Type: |
Domain |
Description: |
The cysteine-rich domain (CRD) is part of the smoothened receptor (Smo), an integral membrane protein and one of the key players in the Hedgehog (Hh) signaling pathway, critical for development, cell growth and migration, as well as stem cell maintenance. The CRD of Smo is conserved in vertebrates and can also be identified in invertebrates. The precise function of the CRD in Smo is unknown []. Mutations in the Drosophila CRD disrupt Smo activity in vivo, while deletion of the CRD in mammalian cells does not seem to affect the activity of overexpressed Smo []. |
|
•
•
•
•
•
|
HT Experiment |
|
Experiment Type: |
RNA-Seq |
Study Type: |
WT vs. Mutant |
Source: |
GEO |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
167
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
89
 |
Fragment?: |
true |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
108
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein Domain |
Type: |
Family |
Description: |
MOSMO, also known as Atthog (attenuator of hedgehog) in mouse, acts as a negative regulator of hedgehog (Hh) signaling, probably by promoting internalization and subsequent degradation of Smoothened (SMO). SMO is an oncoprotein that transduces the Hh signal across the membrane. In the absence of Atthog, SMO was stabilized at the cell surface and concentrated in the ciliary membrane []. |
|
•
•
•
•
•
|
GO Term |
|
•
•
•
•
•
|
Publication |
First Author: |
Malbon CC |
Year: |
2004 |
Journal: |
Front Biosci |
Title: |
Frizzleds: new members of the superfamily of G-protein-coupled receptors. |
Volume: |
9 |
|
Pages: |
1048-58 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ruat M |
Year: |
2013 |
Journal: |
Med Sci (Paris) |
Title: |
[Structure of the Smoothened receptor]. |
Volume: |
29 |
Issue: |
10 |
Pages: |
855-60 |
|
•
•
•
•
•
|
Protein Domain |
Type: |
Domain |
Description: |
Smoothened (SMO) is a transmembrane G protein-coupled receptor that acts as the transducer of the hedgehog (HH) signaling pathway []. SMO is activated by the hedgehog (HH) family of proteins acting on the 12-transmembrane domain receptor patched (PTCH), which constitutively inhibits SMO. Thus, in the absence of HH proteins, PTCH inhibits SMO signaling. On the other hand, binding of HH to the PTCH receptor activates its internalization and degradation, thereby releasing the PTCH inhibition of SMO. This allows SMO to trigger intracellular signaling and the subsequent activation of the Gli family of zinc finger transcriptional factors and induction of HH target gene expression (PTCH, Gli1, cyclin, Bcl-2, etc) []. SMO is closely related to the frizzled (FZD) family of seven transmembrane-spanning proteins, which constitute a novel and separate family of G-protein coupled receptors [, ]. |
|
•
•
•
•
•
|
Publication |
First Author: |
Zhang S |
Year: |
2022 |
Journal: |
Theranostics |
Title: |
The PROTAC selectively degrading Bcl-x(L) represents a novel Hedgehog pathway inhibitor with capacity of combating resistance to Smoothened inhibitors while sparing bone growth. |
Volume: |
12 |
Issue: |
17 |
Pages: |
7476-7490 |
|
•
•
•
•
•
|
Allele |
Name: |
transgene insertion #, Frederic J de Sauvage |
Allele Type: |
Transgenic |
Attribute String: |
Humanized sequence, Inserted expressed sequence |
|
•
•
•
•
•
|
Allele |
Name: |
transgene insertion 199, James M Olson |
Allele Type: |
Transgenic |
Attribute String: |
Constitutively active, Inserted expressed sequence |
|
•
•
•
•
•
|
Strain |
Attribute String: |
coisogenic, endonuclease-mediated mutation, mutant strain |
|
•
•
•
•
•
|
Publication |
First Author: |
Rodriguez-Blanco J |
Year: |
2019 |
Journal: |
Clin Cancer Res |
Title: |
A CK1α Activator Penetrates the Brain and Shows Efficacy Against Drug-resistant Metastatic Medulloblastoma. |
Volume: |
25 |
Issue: |
4 |
Pages: |
1379-1388 |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
337
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
701
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Publication |
First Author: |
Chen Y |
Year: |
1998 |
Journal: |
Development |
Title: |
In vivo evidence that Patched and Smoothened constitute distinct binding and transducing components of a Hedgehog receptor complex. |
Volume: |
125 |
Issue: |
24 |
Pages: |
4943-8 |
|
•
•
•
•
•
|
Publication |
First Author: |
Swiderska-Syn M |
Year: |
2022 |
Journal: |
Sci Adv |
Title: |
Noncanonical activation of GLI signaling in SOX2+ cells drives medulloblastoma relapse. |
Volume: |
8 |
Issue: |
29 |
Pages: |
eabj9138 |
|
•
•
•
•
•
|
Allele |
Name: |
transgene insertion 1, Andrew P McMahon |
Allele Type: |
Transgenic |
Attribute String: |
Inserted expressed sequence |
|
•
•
•
•
•
|
Strain |
Attribute String: |
coisogenic, targeted mutation |
|
•
•
•
•
•
|
Strain |
Attribute String: |
transgenic, coisogenic |
|
•
•
•
•
•
|
Publication |
First Author: |
Hatton BA |
Year: |
2008 |
Journal: |
Cancer Res |
Title: |
The Smo/Smo model: hedgehog-induced medulloblastoma with 90% incidence and leptomeningeal spread. |
Volume: |
68 |
Issue: |
6 |
Pages: |
1768-76 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ward SA |
Year: |
2017 |
Journal: |
Cancer Res |
Title: |
Reprogramming Medulloblastoma-Propagating Cells by a Combined Antagonism of Sonic Hedgehog and CXCR4. |
Volume: |
77 |
Issue: |
6 |
Pages: |
1416-1426 |
|
•
•
•
•
•
|
Publication |
First Author: |
Kim J |
Year: |
2020 |
Journal: |
Proc Natl Acad Sci U S A |
Title: |
Engineered biomimetic nanoparticle for dual targeting of the cancer stem-like cell population in sonic hedgehog medulloblastoma. |
Volume: |
117 |
Issue: |
39 |
Pages: |
24205-24212 |
|
•
•
•
•
•
|
Publication |
First Author: |
Malhotra A |
Year: |
2016 |
Journal: |
Mol Cancer Res |
Title: |
Sonic Hedgehog Signaling Drives Mitochondrial Fragmentation by Suppressing Mitofusins in Cerebellar Granule Neuron Precursors and Medulloblastoma. |
Volume: |
14 |
Issue: |
1 |
Pages: |
114-24 |
|
•
•
•
•
•
|
Publication |
First Author: |
Northcott PA |
Year: |
2009 |
Journal: |
Cancer Res |
Title: |
The miR-17/92 polycistron is up-regulated in sonic hedgehog-driven medulloblastomas and induced by N-myc in sonic hedgehog-treated cerebellar neural precursors. |
Volume: |
69 |
Issue: |
8 |
Pages: |
3249-55 |
|
•
•
•
•
•
|
Publication |
First Author: |
Snuderl M |
Year: |
2013 |
Journal: |
Cell |
Title: |
Targeting placental growth factor/neuropilin 1 pathway inhibits growth and spread of medulloblastoma. |
Volume: |
152 |
Issue: |
5 |
Pages: |
1065-76 |
|
•
•
•
•
•
|
Publication |
First Author: |
Knight ER |
Year: |
2015 |
Journal: |
Oncogene |
Title: |
ASC deficiency suppresses proliferation and prevents medulloblastoma incidence. |
Volume: |
34 |
Issue: |
3 |
Pages: |
394-402 |
|
•
•
•
•
•
|
Publication |
First Author: |
Zhang Y |
Year: |
2018 |
Journal: |
Cancer Res |
Title: |
Nkx2-2as Suppression Contributes to the Pathogenesis of Sonic Hedgehog Medulloblastoma. |
Volume: |
78 |
Issue: |
4 |
Pages: |
962-973 |
|
•
•
•
•
•
|
Publication |
First Author: |
Singh AR |
Year: |
2016 |
Journal: |
PLoS One |
Title: |
PI-3K Inhibitors Preferentially Target CD15+ Cancer Stem Cell Population in SHH Driven Medulloblastoma. |
Volume: |
11 |
Issue: |
3 |
Pages: |
e0150836 |
|
•
•
•
•
•
|
Publication |
First Author: |
Xin Y |
Year: |
2020 |
Journal: |
Sci Rep |
Title: |
PET imaging of medulloblastoma with an 18F-labeled tryptophan analogue in a transgenic mouse model. |
Volume: |
10 |
Issue: |
1 |
Pages: |
3800 |
|
•
•
•
•
•
|
Publication |
First Author: |
Veiseh O |
Year: |
2009 |
Journal: |
Cancer Res |
Title: |
Specific targeting of brain tumors with an optical/magnetic resonance imaging nanoprobe across the blood-brain barrier. |
Volume: |
69 |
Issue: |
15 |
Pages: |
6200-7 |
|
•
•
•
•
•
|
Publication |
First Author: |
Wei Y |
Year: |
2019 |
Journal: |
Mol Cancer Res |
Title: |
p53 Function Is Compromised by Inhibitor 2 of Phosphatase 2A in Sonic Hedgehog Medulloblastoma. |
Volume: |
17 |
Issue: |
1 |
Pages: |
186-198 |
|
•
•
•
•
•
|
Allele |
Name: |
transgene insertion, James M Olson |
Allele Type: |
Transgenic |
Attribute String: |
Constitutively active, Inserted expressed sequence |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Tg(Neurod2-Smo*A1)199Jols/? |
Background: |
C57BL/6-Tg(Neurod2-Smo*A1)199Jols |
Zygosity: |
ot |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Tg(Neurod2-Smo*A1)199Jols/Tg(Neurod2-Smo*A1)199Jols |
Background: |
C57BL/6-Tg(Neurod2-Smo*A1)199Jols |
Zygosity: |
hm |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Tg(Neurod2-Smo*A1)199Jols/? |
Background: |
involves: C57BL/6 |
Zygosity: |
ot |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
689
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Publication |
First Author: |
Castellino RC |
Year: |
2010 |
Journal: |
PLoS One |
Title: |
Heterozygosity for Pten promotes tumorigenesis in a mouse model of medulloblastoma. |
Volume: |
5 |
Issue: |
5 |
Pages: |
e10849 |
|
•
•
•
•
•
|
Strain |
Attribute String: |
coisogenic, mutant strain, transgenic |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Tg(Col2a1-GAL4)1Amc/? Tg(UAS-Smo*W539L)1Amc/? |
Background: |
Not Specified |
Zygosity: |
cx |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Tg(Neurod2-Smo*A2)#Jols/? |
Background: |
C57BL/6-Tg(Neurod2-Smo*A2)#Jols |
Zygosity: |
ot |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Publication |
First Author: |
Monkkonen T |
Year: |
2017 |
Journal: |
Development |
Title: |
Epithelial and non-epithelial Ptch1 play opposing roles to regulate proliferation and morphogenesis of the mouse mammary gland. |
Volume: |
144 |
Issue: |
7 |
Pages: |
1317-1327 |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
418
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
410
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Publication |
First Author: |
Huang HC |
Year: |
2004 |
Journal: |
Genome Biol |
Title: |
The Frizzled family: receptors for multiple signal transduction pathways. |
Volume: |
5 |
Issue: |
7 |
Pages: |
234 |
|
•
•
•
•
•
|
HT Experiment |
Series Id: |
GSE17473 |
Experiment Type: |
transcription profiling by array |
Study Type: |
Baseline |
Source: |
ArrayExpress |
|
•
•
•
•
•
|
Publication |
First Author: |
Bhatia B |
Year: |
2011 |
Journal: |
Oncogene |
Title: |
Mitogenic Sonic hedgehog signaling drives E2F1-dependent lipogenesis in progenitor cells and medulloblastoma. |
Volume: |
30 |
Issue: |
4 |
Pages: |
410-22 |
|
•
•
•
•
•
|
Publication |
First Author: |
Abad C |
Year: |
2014 |
Journal: |
J Leukoc Biol |
Title: |
Targeted STAT3 disruption in myeloid cells alters immunosuppressor cell abundance in a murine model of spontaneous medulloblastoma. |
Volume: |
95 |
Issue: |
2 |
Pages: |
357-67 |
|
•
•
•
•
•
|
Publication |
First Author: |
Bhatia S |
Year: |
2015 |
Journal: |
Oncotarget |
Title: |
Knockdown of EphB1 receptor decreases medulloblastoma cell growth and migration and increases cellular radiosensitization. |
Volume: |
6 |
Issue: |
11 |
Pages: |
8929-46 |
|
•
•
•
•
•
|
Publication |
First Author: |
Paine MRL |
Year: |
2019 |
Journal: |
Sci Rep |
Title: |
Three-Dimensional Mass Spectrometry Imaging Identifies Lipid Markers of Medulloblastoma Metastasis. |
Volume: |
9 |
Issue: |
1 |
Pages: |
2205 |
|
•
•
•
•
•
|
Publication |
First Author: |
Michael LE |
Year: |
2008 |
Journal: |
Neoplasia |
Title: |
Bmi1 is required for Hedgehog pathway-driven medulloblastoma expansion. |
Volume: |
10 |
Issue: |
12 |
Pages: |
1343-9, 5p following 1349 |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Mir34a/Mir34a<+> Tg(Neurod2-Smo*A1)199Jols/? |
Background: |
involves: C57BL/6 * C57BL/6N |
Zygosity: |
cx |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
Genotype |
Symbol: |
Mir34a/Mir34a Tg(Neurod2-Smo*A1)199Jols/? |
Background: |
involves: C57BL/6 * C57BL/6N |
Zygosity: |
cx |
Has Mutant Allele: |
true |
|
•
•
•
•
•
|
HT Experiment |
Series Id: |
GSE11063 |
Experiment Type: |
transcription profiling by array |
Study Type: |
WT vs. Mutant |
Source: |
ArrayExpress |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
447
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
298
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
293
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
299
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
295
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
299
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
313
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
311
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
215
 |
Fragment?: |
true |
|
•
•
•
•
•
|
Publication |
First Author: |
Aarts MG |
Year: |
1995 |
Journal: |
Plant Cell |
Title: |
Molecular characterization of the CER1 gene of arabidopsis involved in epicuticular wax biosynthesis and pollen fertility. |
Volume: |
7 |
Issue: |
12 |
Pages: |
2115-27 |
|
•
•
•
•
•
|
Publication |
First Author: |
Darnet S |
Year: |
2003 |
Journal: |
Biochim Biophys Acta |
Title: |
Enzymological properties of sterol-C4-methyl-oxidase of yeast sterol biosynthesis. |
Volume: |
1633 |
Issue: |
2 |
Pages: |
106-17 |
|
•
•
•
•
•
|
Publication |
First Author: |
Husselstein T |
Year: |
1999 |
Journal: |
Plant Mol Biol |
Title: |
Delta7-sterol-C5-desaturase: molecular characterization and functional expression of wild-type and mutant alleles. |
Volume: |
39 |
Issue: |
5 |
Pages: |
891-906 |
|
•
•
•
•
•
|
Publication |
First Author: |
Darnet S |
Year: |
2004 |
Journal: |
Biochem J |
Title: |
Plant sterol biosynthesis: identification of two distinct families of sterol 4alpha-methyl oxidases. |
Volume: |
378 |
Issue: |
Pt 3 |
Pages: |
889-98 |
|
•
•
•
•
•
|
Publication |
First Author: |
Zhu G |
Year: |
2015 |
Journal: |
J Biol Chem |
Title: |
The Crystal Structure of an Integral Membrane Fatty Acid α-Hydroxylase. |
Volume: |
290 |
Issue: |
50 |
Pages: |
29820-33 |
|
•
•
•
•
•
|
Protein Domain |
Type: |
Domain |
Description: |
This entry includes fatty acid and carotene hydroxylases and sterol desaturases. Beta-carotene hydroxylase is involved in zeaxanthin synthesis by hydroxylating beta-carotene, but the enzyme may be involved in other pathways []. This family includes C-5 sterol desaturase []and C-4 sterol methyl oxidase (SMO) [, ]. Members of this family are involved in cholesterol biosynthesis and biosynthesis a plant cuticular wax. These enzymes contain two copies of a HXHH motif, involved in coordination of two iron atoms in the catalytic centre. Members of this family are ER integral membrane proteins with a mushroom-like structure consisting of four transmembrane helices (TM1-TM4) that anchor them to the membrane, capped by a cytosolic domain containing the unique histidine-coordinating di metal centre [].The plant SMO amino acid sequences possess three histidine-rich motifs (HX3H, HX2HH and HX2HH), characteristic of the small family of membrane-bound non-haem iron oxygenases that are involved in lipid oxidation []. |
|
•
•
•
•
•
|
Publication |
First Author: |
Wang GY |
Year: |
2011 |
Journal: |
Cancer Cell |
Title: |
Basal cell carcinomas arise from hair follicle stem cells in Ptch1(+/-) mice. |
Volume: |
19 |
Issue: |
1 |
Pages: |
114-24 |
|
•
•
•
•
•
|
Publication |
First Author: |
Klisch TJ |
Year: |
2017 |
Journal: |
Elife |
Title: |
Jak2-mediated phosphorylation of Atoh1 is critical for medulloblastoma growth. |
Volume: |
6 |
|
|
|
•
•
•
•
•
|
Publication |
First Author: |
Pokrajac NT |
Year: |
2022 |
Journal: |
Commun Biol |
Title: |
Three dimensional reconstruction of the mouse cerebellum in Hedgehog-driven medulloblastoma models to identify Norrin-dependent effects on preneoplasia. |
Volume: |
5 |
Issue: |
1 |
Pages: |
569 |
|
•
•
•
•
•
|
Publication |
First Author: |
Hatton BA |
Year: |
2010 |
Journal: |
Oncogene |
Title: |
Notch signaling is not essential in sonic hedgehog-activated medulloblastoma. |
Volume: |
29 |
Issue: |
26 |
Pages: |
3865-72 |
|
•
•
•
•
•
|
Publication |
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